Primary lymphoma of the uterine corpus and cervix is rare. We present a case of primary non-Hodgkin follicular lymphoma isolated to uterine corpus and parametria with focal spread to ovaries and fallopian tubes, incidentally found on the background of endometrial malignancy. A summary of the published cases focusing on the presentation and prognosis as well as a review of current management are discussed. The rising incidence of extra-nodal lymphoma and recent changes in classification and therapeutic approach, require clinical vigilance. In the absence of prospective studies assessing the value of the available therapeutic options, data from retrospective series and scattered case reports are presented in this review.